The prognosis of primary lymphedema of the lower limbs.

Primary lymphedema in most patients can be managed conservatively, but, in a few, severe lymphedema develops that requires surgical reduction of the limb. The five- to 20-year course of the disease has been studied in 372 patients. The eventual extent of the disease could be assessed within the first year. Further spreading of the lymphedema in the same limb occurred in only 7% of the patients after the first year and in less than 1% after five years. Only 9% of the patients suffered lymphedema of the other leg at a later time. Patients without pelvic lymphatic obstruction had a good prognosis even if the distal lymphatics were obliterated, but 32% of those with proximal obstructive hypoplasia had severe lymphedema that required surgical reduction. In this latter group, men and women were affected equally.

Operations for lymphoedema of the lower limbs. A study of the results in 108 operations utilizing vascularized dermal flaps.

The results of operations for lymphoedema of the lower limbs have been reviewed. In experienced hands the results are very satisfactory. Modifications of the buried dermis flap operation of Thompson have been found to give the best results in patients where the local skin has been in good condition. The chief complication has been the occurrence of localized areas of necrosis, chiefly at the edges of the flaps. It has not been a serious complication but has sometimes prolonged the stay in hospital. It has been associated with haematoma formation. Methods of avoiding this and other complications are discussed in order to improve results still further. There have been no serious complications and no deaths in this series.

Protein-losing enteropathy in lymphoedema. Surgical investigation and treatment.

Patients with primary lymphoedema have been tested for protein loss from the gut by the chromium chloride test. In a proportion of patients there is significant loss which may drop the plasma proteins thereby increasing oedema. Three patients were treated by abdominal exploration, mesenteric lymphography and resection of the worst affected length of gut. There was clinical and biochemical improvement and no complication.

A gut and mesentery pedicle for bridging lymphatic obstruction: experimental studies.

Experiments have been carried out in the pig. Small bowel and its mesentery are rich in lymphatics. A pedicle of ileum (opened and denuded of its mucosa) and its mesentery has been used to bypass lymphatic obstruction. Diversion of lymphatic drainage from the lower limb into the lymphatics of the bridge has been demonstrated lymphographically in the living animals and also by post mortem injection studies.

Lymphatic obstruction and lymph node changes--a study of the rabbit popliteal node.

Dense cortical lymph node fibrosis is associated with primary lymphedema and proximal obstructive hypoplasia. The fibrosis is not related to the duration of disease nor to episodes of clinical cellulitis. This suggests that the disease may start in the nodes but the effects of obstruction in the adjacent lymph vessel must be elucidated before the assumption is made. The popliteal nodes of 49 rabbits were studied following the ligation of either afferent or efferent lymphatics, and compared with nodes following a sham operation. The efficacy and late effects of ligation were assessed in half the rabbits by cinelymphangiography. In the rest Patent Blue Violet and not Lipoodol was used to minimize any spurious effect on node histology. The rabbits were sacrificed between 6-84 days. Node area was measured on serial x-rays and node volume after removal of the node. Afferent ligation resulted in a significant and permanent decrease in size (p less than 0.005) and lymphocytic congestion; efferent ligation resulted in an increase in size (p less than 0.02) and large lymph spaces. The dense node fibrosis of primary lymphedema was not seen.

Comments on operations for lower limb lymphoedema.

Good results have been obtained from surgical operations for lymphoedema of the lower limbs using skin flaps with a blood supply to cover the muscles following reduction of swollen subcutaneous tissue. The Charles operation using free skin grafts for cover is reserved for tropical elephantiasis or patients with local skin in bad condition. A variety of other procedures of physiologic intent have given disappointing results and been abandoned. The results of 74 operations are reviewed with a view to improving still further the results. The mortality rate was nil and there were only minor complications.

"Die-back" in primary lymphedema -- lymphographic and clinical correlations.

Both obliteration and dilatation of distal lymphatics are seen in primary lymphedema but it is only in secondary lymphedema that the lymphographic progression of the disease has been documented. In this retrospective study the same progression was found in 13 out of 20 patients had initial evidence of proximal lymphatic obstruction. In ten patients the lymphangiographic changes were associated with a clinical deterioration of the leg. These findings suggest some urgency in diagnosing those patients that may benefit from a lymph bypass procedure.

The distribution of an oil-based corticosteroid following intralymphatic infusion.

Following infusion of tritium labeled clobetasol propionate in Ultrafluid Lipidol (UFL) into a right hind limb lymphatic of rabbits, the radioactivity levels in various tissues at intervals up to 28 days were determined by liquid scintillation counting. There was a rapid decline in activity in the right popliteal node over the first three days due to early bloodstream absorption. From three to 28 days radioactivity levels were consistently higher in the right popliteal node and lung than in other tissues sampled. This distribution suggests that there is an affinity between clobetasol and the lipidol vehicle which retards (but does not prevent) free diffusion of this agent out of lymphatic tissues. Thus, while permitting generalized perfusion of tissues by clobetasol propionate, intralymphatic infusion maximizes its initial concentration and duration of activity within specific node groups and, therefore, may be useful in certain patients with primary lymphedema where lymph nodes affected by fibrosis constitute a major site of obstruction.

Intralymphatic steroid therapy for lymphoedema: preliminary studies.

Lymph node fibrosis is a significant feature in patients with primary lymphoedema. Its importance in causing obstruction to the flow of lymph has lead to this study which is an attempt to reduce the effect of fibrosis by treatment with a corticosteroid preparation administered by direct intralymphatic infusion. In a pilot study, twenty patients received an intralymphatic infusion of Clobetasol propionate in Ultra-fluid Lipiodol (18 lower limb, 2 face). Clinical improvement occurred in eight of the patients with leg oedema over a period of up to nine months. Both face-affected patients were helped by this form of therapy. In patients with lymphoedema of the lower limb with lymph node abnormalities, it was advantageous if the lymph vessels leading up to the nodes were patent.

Lymph nodes and vessels in primary lymphoedema. Their relative importance in aetiology.

The classification of lymphoedemas is reviewed and the various primary lymphoedemas distinguished from the secondary. The early lymphographic studies of primary lymphoedemas (many of which arise from genetic factors) probably laid undue emphasis on changes in the lymphatic vessels. Oil contrast media in leter use in lymphography have given much information onchanges in the nodes. In 89% of patients reviewed both nodes and vessels were diseased and in the majority the changes were worse in the nodes. This suggests that in many patients with primary hypoplastic lymphoedema the pathological process has arisen first in the nodes.

Fibrosis in the lymph nodes in primary lymphoedema. Histological and clinical studies in 74 patients with lower-limb oedema.

There are many different clinical and lymphographic groups of patients with primary lymphoedema. Improved lymphographic techniques have emphasised the importance of changes in the nodes as well as in the lymph vessels. A systematic histological study has been made of nodes removed during therapeutic operations or investigations on patients with primary lymphoedema. Many nodes showed a marked fibrotic process. This, in its distribution in the node and its histological appearance, was quite different from that which might have arisen from attacks of infection and inflammation. The majority of patients had no clinical history of such attacks. It may be regarded as primary fibrosis in the nodes. Associated clinical features suggest strong genetic or familial factors in its aetiology. Many of the changes found in the lymph vessels may follow obstructive effects from fibrosis in the nodes. The histological findings have been related to the clinical and lymphographic features in different types of primary lymphoedema. The degree of fibrosis and its distribution have important bearings on the prognosis, clinical course, and treatment of the patient.

Pretibial myxoedema.

This paper reviews the clinical, endocrine and lymphatic status of 6 patients with pretibial myxoedema. The lymphatics were studied to determine whether they were involved in the pathogenesis of the disease. The main lymph trunks were normal although there was occasionally some collateral flow and some of the lymph nodes had small irregular filling defects. These changes suggest compression of the lymphatics by the myxomatous material and the deposition of mucinous material in the lymph nodes. There was no evidence of a primary lymphatic abnormality.